Abstract
Refractory celiac disease is a rare complex autoimmune-mediated disorder, which has multiple complications, among the best known is enteropathy-associated T-cell lymphoma, other rare complications are central nervous system demyelinating disorder and cavitary mesenteric lymph node syndrome. In our case report, we present a 43-year-old female with a known history of refractory celiac disease who developed a progressive demyelinating disorder of unknown origin and elevated liver enzymes that warranted further investigation. This led to the diagnosis of the cavitary mesenteric lymph node syndrome and celiac disease associated with demyelinating disorder that are both very rare presentations of refractory celiac disease. Treatment options for this case presented a challenge, firstly due to the lack of literature but also to continued complications.